[Olivopontocerebellar atrophy--a heterogenous morphologic syndrome].
Among the neuropathologically defined spinocerebellar degenerations, olivopontocerebellar atrophy (OPCA) represents a morphologic syndrome underlying different clinical, genetic, and biochemical conditions. Clinical and genetic classification systems include OPCA in sporadic cerebellar ataxias such as multiple system atrophy as well as in different autosomal recessive, autosomal dominant and X-chromosomal recessive cerebellar ataxias. According to findings of the last two decades, the historically derived term OPCA should be avoided in clinical use and should be regarded as a diagnostic contribution. A proper diagnostic classification of individual cases, however, has to consider all neuropathological, clinical and genetic data.
St Probst-Cousin
Institut für Neuropathologie, Westfälische Wilhelms-Universität, Münster.
D006801:Humans; D009849:Olivopontocerebellar Atrophies / Q000473:pathology* / Q000503:physiopathology / Q000523:psychology
Die olivopontozerebelläre Atrophie--ein heterogenes morphologisches Syndrom.